Abstract
INTRODUCTION AND IMPORTANCE: Primary pulmonary artery sarcoma (PPAS) is a rare malignant tumor composed of malignant tumor tissue originating from pulmonary artery endothelial cells and smooth muscle cells. The etiology of the disease is unknown and the clinical manifestations are insidious. PPAS is extremely rare in clinical practice, with difficult preoperative diagnosis, high misdiagnosis rate and poor prognosis. CASE PRESENTATION: This article reports a case of a patient who had previously undergone mitral valve replacement surgery and presented symptoms of chronic pulmonary artery embolism. The patient underwent pulmonary artery endarterectomy, and intraoperative and postoperative pathological examination results showed that the patient had PPAS. CLINICAL DISCUSSION: In clinical practice, when excluding patients with high-risk factors for deep vein thrombosis, normal D-dimer levels, and ineffective anticoagulant therapy in patients with suspected pulmonary embolism, PPAS should be considered as a possibility. CONCLUSION: After surgical treatment, the patient successfully recovered and was discharged. One year of follow-up has shown that the patient's general condition is good.